摘要目的:探讨高免疫球蛋白E(IgE)血症患儿临床疾病谱的特点。方法:选择2012年1月至2018年12月中山大学孙逸仙纪念医院儿科收治的血清总IgE≥5×10 5 IU/L的498例患儿为研究对象。回顾性分析498例患儿的临床资料，总结其病因分布、临床表现、实验室检查结果、治疗及转归等情况。并根据血清总IgE水平高低分为IgE轻度升高组(5×10 5~<10×10 5 IU/L)、中度升高组(10×10 5~<20×10 5 IU/L)、重度升高组(≥20×10 5 IU/L)，分析比较3组患儿疾病谱的分布情况。 结果:(1)疾病谱中病因以过敏性疾病最常见(213例)，其次为感染性疾病(163例)；病原体以肺炎支原体(109例)及EB病毒(120例)感染多见。(2)轻度升高组中过敏性疾病(45.0%)、感染性疾病(42.2%)的发生率高于中度升高组(40.8%、26.2%)和重度升高组(38.9%、12.2%)，差异均有统计学意义(均 P<0.001)；中度升高组中免疫性疾病(18.5%)、肿瘤及血液系统疾病(5.4%)的发生率均较轻度升高组高(4.4%、2.0%)，差异均有统计学意义(均 P<0.001)，重度升高组中免疫性疾病(34.4%)、肿瘤及血液系统疾病(11.1%)发生率均较轻度升高组(4.4%，2.0%)、中度升高组(18.5%、5.4%)高，差异均有统计学意义(均 P<0.001)。(3)临床表现以发热(63.5%)、呼吸道症状(53.7%)及淋巴结大(53.7%)为主；47.5%的患儿外周血白细胞计数升高，12.1%的患儿嗜酸性粒细胞计数升高。(4)特异性IgE测定最常见的过敏原为尘螨组合(32.0%)、牛奶(17.0%)、鸡蛋白(16.0%)。特异性IgE阳性的患儿中总IgE水平轻度升高组、中度升高组、重度升高组的病种分布比较差异无统计学意义( P＝0.164)。 结论:儿童高IgE血症的病因以过敏性疾病、感染性疾病最常见，轻中重度高IgE血症患儿的病种分布比较差异有统计学意义，其总IgE水平越高，风湿性疾病、免疫缺陷病、肿瘤及血液系统疾病的发生率越高。

abstractsObjective:To investigate the clinical characteristics of the disease spectrum of pediatric hyper blood immunoglobulin E (IgE).Methods:A total of 498 children with total serum IgE ≥ 5×10 5 IU/L admitted to the Department of Pediatrics, Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University from January 2012 to December 2018 were enrolled.Their clinical data, etiology distribution, clinical manifestations, laboratory results, treatment and outcomes were retrospectively analyzed.According to serum total IgE level, patients were divided into mildly increased IgE group (5×10 5-<10×10 5 IU/L), moderately increased group (10×10 5-<20×10 5 IU/L), and severely increased group (≥20×10 5 IU/L). The distribution of disease types among the 3 groups were compared. Results:(1) Allergic disease (213 cases) was the most common etiology in children with hyper blood IgE, and infectious disease (163 cases), mycoplasma pneumoniae (109 cases) and EB virus (120 cases) were common pathogens.(2) The incidence of allergic diseases (45.0%) and infectious diseases (42.2%) in the mildly increased group was significantly higher than that in the moderately increased group (40.8%, 26.2%, respectively) and the severely increased group(38.9%, 12.2%, respectively) (all P<0.001). The incidence of immune diseases(18.5%), tumors and hematological diseases (5.4%) in the moderately increased group was significantly higher than that of the mildly increased group (4.4%, 2.0%, respectively) (all P<0.001). The incidence of immune diseases (34.4%), tumors and hematological diseases (11.1%) in the severely increased group was significantly higher than that of the mildly increased group(4.4%, 2.0%, respectively) and the moderately increased group (18.5%, 5.4%, respectively) (all P<0.001). (3) The main clinical manifestations were fever (63.5%), respiratory symptoms (53.7%) and lympha-denopathy (53.7%), 47.5% of the children with hyper blood IgE had an increased white blood cell count, and 12.1% of them had an increased eosinophil count.(4) The most common specific allergens were dust mite combination (32.0%), milk (17.0%), and egg white (16.0%). There was no difference in disease distribution among the 3 groups of hyper blood IgE children with positive specific IgE ( P=0.164). Conclusions:Hyper blood IgE in children are most commonly caused by allergic and infectious diseases.The etiological distributions of hyper blood IgE in children at varying severities differ a lot.The higher the total IgE level, the higher the incidence of immunodeficiency disease, rheumatic disease, tumor and hematological disease.